Flashcards in Tricuspid Valve Anomalies Deck (23):
What is TV atresia?
A cyanotic lesion with complete failure of the TV formation. There is only a thickened annulus/membrane.
With TV atresia what happens to the RV?
It is completely dysfunctional and hypoplastic (small and underdeveloped). Pts with this defect are considered to only have one ventricle (the LV).
What other defects are associated with TV atresia, and why would they be important for a baby born with it to have?
ASD, VSD, & PDA. It is important for an infant to have these shunts to ensure blood goes to the lungs, otherwise it isn't compatible with life. Without these the Pt will go into respiratory distress. There may also be transposition of the great vessels.
What happens to flow in a Pt with TV atresia?
There is venous and arterial mixing. This causes 02 sats to be low. The Pt will be cyanotic and have poor gas exchange.
What is the most common type of TV atresia?
No transposition of the great vessels, with a small ASD & VSD.
What are the symptoms of TV atresia?
- Marked cyanosis at birth
- Increased crying which can cause cerebral hypoxia
- Rapid breathing & increased HR
- Pulmonary HTN
- Respiratory distress
- Baby will be intubated right away
What is the treatment for TV atresia?
There will be 3 surgeries over the first 2 years once the baby is stable in NICU.
What is balloon septostomy and why is it done?
It is the creation of an ASD with a catheter. It is needed to allow blood flow to reach the lungs.
What is ProstagladinE used for?
It is used to keep the PDA open by keeping the tissue relaxed and prevent it from growing.
What are the names for the 3 surgeries used to correct TV atresia?
1st surgery: Blalock-Tausigg (BT)
2nd surgery: Glenn shunt
3rd surgery: Fontane procedure
What is a Blalock- Tausigg shunt?
It is a shunt created by attaching the right subclavian artery to the right pulmonary artery using a graft.
What is a Glenn shunt?
It is a graft that re-routes blood flow to bypass the RA. Blood flows from the SVC directly to the PA and then to the lungs in order to get oxygen.
What is the Fontane procedure?
It is the complete separation of the systemic from the pulmonary circulation. The IVC is connected to the right pulmonary artery.
How is the IVC connected to the RPA in the Fontane procedure?
Either by a tunnel-like patch within the RA to redirect flow to RPA, or by a direct communication outside the heart from the IVC to the RPA.
After the Fontane procedure is completed what else will be done?
Any ASD & VSD will be closed. There may be PA banding if necessary and the coronary sinus will be redirected.
What is Ebsteins malformation of the TV?
A cyanotic lesion where the TV is displaced inferiorly towards the apex.
What happened during fetal development to cause Ebsteins?
The leaflets of the TV never separated from the myocardium. The leaflets become abnormal.
What happens to the RV in a Pt with Ebsteins?
Portion of the RV becomes atrialized and it becomes too small and hypertrophies leading to disfunction. The RA will become huge.
What happens to the pulmonary circulation with Ebsteins?
There will be pulmonary HTN and increased pulmonary vascular resistance. There may also be TR due to the volume overload and increased pressures.
What are some other lesions associated with Ebsteins?
1. PDA with ASD & VSD
2. PA atresia due to narrowed PA and RVOT obstruction
3. Pulmonary stenosis
4. Tetralogy of Fallot
What is the clinical presentation of Ebsteins?
- If it is severe the Pt will show signs of CHF
- Respiratory distress and HTN.
- Murmurs: TR, PS, P2 is delayed with palpable thrill
- Arrhythmia's: PACs and STach
- May hear S4 heart sound with right heart failure
What are the surgical repairs for Ebsteins?
Repair or replacement of the TV with a tissue valve. Also close any ASD or VSD and correct any other CHDs.